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Steve Jobs, dead at 56, had a rare form of pancreatic cancer

Steve Jobs arrives with the team from the best-picture-nominated film… (Lucas Jackson / Reuters )

Apple co-founder Steve Jobs, who passed away today at the age of 56, had a rare form of pancreatic cancer called pancreatic neuroendocrine cancer, which produces islet cell or neuroendocrine tumors.

This form is usually less aggressive than pancreatic exocrine cancer and patients can live longer, with the average survival rate more than three years. Some people with the neuroendocrine form can live as long as 20 years.

Several forms of treatment are available. Jobs was diagnosed in 2003, had a liver transplant in 2009 and took an extended medical leave from Apple last January.

During his public appearances before he retired as Apple's CEO in August, it was evident Jobs had lost a significant amount of weight.

“Weight loss when it comes to advanced cancer is never a good thing,” said Dr. Jack Jacoub, a medical oncologist at MemorialCare Cancer Institute at Orange Coast Memorial Medical Center in Fountain Valley. Keeping body weight up and healthy while fighting the disease is key, he added. “We use 10% weight loss as being a negative prognosticator.”

There’s a propensity for the disease to spread from the pancreas to the liver, Jacoub said -- which is why many local treatments focus on attacking cancer cells in the liver, including destroying them with heat or surgically removing them.

In some cases, removing a diseased liver entirely and replacing it with a donor liver is an option -- but liver transplants aren’t that common among people with this type of pancreatic cancer, Dr. Craig Devoe said in August. Devoe is an oncologist at the North Shore-LIJ Health System in New Hyde Park, N.Y., who specializes in pancreatic cancer. But a transplant can be done when other options have run out or when the disease has spread to the liver. It’s not typically thought of as a cure.

Even after a liver transplant, the cancer can recur, Jacoub said, which may have been what happened in Jobs' case. “Probably nine out of 10 times it’s the recurrence of the cancer, but it’s also possible that he suffered from the toxicity of the drugs that he was taking,” Jacoub said. Infections can also occur because the drugs keeping the body from rejecting the liver transplant suppress the immune system.

Neuroendocrine tumors can grow slowly; ones that are functional can secrete hormones and cause symptoms such as stomach ulcers, high blood sugar or skin rashes. Nonfunctioning tumors can grow without being noticed, and they don't produce hormones or symptoms.

The disease is sometimes treated like a condition, much like diabetes. Therapies include medication and low-toxic oral chemotherapy. Two drugs, Sutent and Afinitor, were shown in two 2011 New England Journal of Medicine studies to slow the progression of tumors. Peptide receptor radiotherapy is another form of treatment in which peptides radiate neuroendocrine tumor cell receptors.

Jobs, Jacoub said, likely had many resources available to fight the cancer, and there was little that could have been done differently. Living in the public eye, Jobs dealt with the disease “in such a graceful way -- you’d be hard pressed to find another person that did it on his terms,” Jacoub said.